CONGENITAL HEPATIC-FIBROSIS ASSOCIATED WITH CAVERNOUS TRANSFORMATION OF THE PORTAL-VEIN

Background/Aims: Congenital hepatic fibrosis (CHF), which is one of th e fibropolycystic diseases, occurs in various forms. Portal hypertensi on, a very common clinical feature of this condition, has been attribu ted to the compression of portal vein radicles in the fibrous bands. W e investigated whether there are any other contributing factors in the development of portal hypertension in patients with CHF. Methodology: A total of 1285 patients with portal hypertension of different etiolo gies were studied using ultrasonography as the screening test. Forty-s even (including portal vein involvement and/or CHF) of these 1285 pati ents were prospectively studied to evaluate the etiology of the portal hypertension by portography, abdominal computed tomography, explorato ry laparotomy, peritonoscopy, liver biopsy and laboratory tests. The p atients with CHF were divided into two groups, according to whether or not they had portal vein involvement. Results: Eleven (0.8%) of the 1 285 patients with portal hypertension had CHF, and 41 (3.2%) had caver nous transformation of the portal vein (CTPV) resulting from different or unknown etiologies. Five patients had both pathologies (CTPV and C HF). In the 11 patients with CHF, there was CTPV in 5 patients, Caroli 's disease in 2 patients, cholangiocarcinoma in 1 patient, inferior ve na caval obstruction in 1 patient, and CHF in only 2 patients. There w ere statistically significant differences in the age of the CHF patien ts at clinical onset, the incidence of bleeding from esophageal varice s, and laboratory findings between the 2 groups with and without CTPV. Despite a thorough investigation we could not distinguish any predisp osing factor in 25 of the 41 patients with CTPV. The incidence of CTPV was 48% in patients with. CHF and 3.2% in patients with portal hypert ension. Conclusions: These results suggest that the association of CTP V with CHF is not coincidental, but that CTPV may be associated with C HF and a new possible factor in portal hypertension, and that it can b e a major factor in the manifestation of esophageal bleeding from vari ces.